Thalassemia is an inherited blood disorder in which parents pass the genes for disorder on to their children. It reduces reproduction of red blood cells in human body. In thalassemia patient, the genes that control globin production are mutated or deleted. As a result, formation of the corresponding globin chains is trimmed down and an abnormal hemoglobin ratio is enhanced. This abnormal ratio leads to a decreased synthesis of hemoglobin and expression of thalassemia.
Professor Dr. Mamun Ahmed from the Department of Biochemistry and Molecular Biology, Dhaka University explained, “If one among 23 pairs of chromosomes of one’s body is affected by Alpha or Beta chain mutation, one may become victim of Thalassemia. If both the chromosomes of a pair are affected, chance is 25 percent that the person will be victim of this lethal disease.”
Thalassemia causes mutation in the DNA of cells that make hemoglobin, the substance in the red blood cells that carries oxygen throughout the body. The mutations thus disrupt the normal production of hemoglobin and cause low hemoglobin levels and a high rate of red blood cell destruction, causing anemia. Due to anemia, the blood does not have enough red blood cells to carry oxygen to the tissues, leaving one fatigued.
Epidemiology
Generally, thalassemia is prevalent in the people living in humid climate zones where malaria was endemic in the past or still exists. Since thalassemia acts as an antidote to malaria because of the blood cells' easy degradation, it got epidemic in those regions.
Thalassemia was first detected among Mediterranean inhabitants. In fact, this geographical association is responsible for its naming: Thalassa is Greek for the sea, Haema is Greek for blood. In Europe, the highest concentrations of the disease are found in Greece, coastal regions of Turkey, Southern Italy and the lower Po valley. The major Mediterranean islands (except the Balearics) such as Sicily, Sardinia, Malta,